抗NMDA受体脑炎45例特点和临床转归分析  

The clinical characteristics and outcomes of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis:a study of 45 cases

作　　者：冯雪丹[1] 胡永强[1] 冯文良 程家玲 张隆辉[1] 邓欢[1] 王琳[2] 

机构地区：[1]北京丰台右安门医院神经内科,北京100069 [2]首都医科大学宣武医院神经内科

出　　处：《脑与神经疾病杂志》2016年第7期425-429,共5页Journal of Brain and Nervous Diseases

摘　　要：目的探讨本组抗NMDA受体脑炎患者的临床特点。方法收集北京丰台右安门医院和北京宣武医院神经内科2012年10月至2015年4月收治的45例抗NMDA受体脑炎患者的临床资料,分析病例特点,随访病情转归。结果本组的45例患者年龄为14~61岁,平均年龄32.2±13.24岁;男性27例,其中3例（11%）合并肿瘤。女性18例,其中5例（28%）合并卵巢畸胎瘤。23例（51%）出现前驱症状。本组45例临床症状表现为精神症状（91%）、癫痫发作（76%）、不自主运动（42%）、中枢性低通气（24%）、意识水平下降（47%）及自主神经功能障碍（40%）。脑脊液（CSF）常规、生化检查阳性率为89%,所有患者CSF抗NMDA受体抗体阳性。脑电图（EEG）多表现为双额、颞、中央导联为主的轻度（21%）至中度（59%）慢波。53%患者头颅CT平扫或MRI检查有异常表现,多见于额颞叶T2-Flair异常信号。所有患者均给予激素和丙种球蛋白治疗,6例患者接受环磷酰胺或吗替麦考酚治疗。除1例失访,44例患者预后良好（MRS评分0-2分）者占86%。结论抗NMDAR脑炎发病男性并不少见,肿瘤合并率低。以精神行为异常、癫痫发作、CSF抗NMDA受体抗体阳性为其主要临床特点。绝大部分患者EEG异常,并与病情严重程度相关。79%患者一线免疫治疗效果良好。8例合并肿瘤患者病情严重且预后不良,畸胎瘤摘除术不能完全预防该病发生,但能减轻病情严重程度。Objective To investigate the clinical characteristics of anti-N-methyl-D-aspartate receptor （NMDAR）encephalitis in the group. Methods The clinical data of 45 cases with anti-NMDAR encephalitis at neurology department of Beijing Youanmen Hospital and Beijing Xuanwu hospital from October 2012 to April 2015 were collected. The clinical features and followed-up outcomes were analyzed retrospectively. Results In the study of 45 patients, the outbreak age was between 14-61 years old, the mean age was 32.2±13.24 years. Twenty-seven patients were male, three of who（11%）had an underlying tumors. Eighteen were female, five of who（28%）had ovarian teratomas. Prodiomal symptoms were noted in 23（51%）of patients. The main clinical manifestations include psychiatric symptoms（91%）, seizures（76%）, involuntary movement（42%）, hypoventilation（24%）, decreased level of consciousness（47%）and autonomic instability（40%）.The CSF routine test and biochemistry panel were abnormal in 89% patients and test for anti-NMDA receptor antibodies in CSF were positive in all patients. The EEG records main show generalized or frontal to temporal slow waves wild mild degree（21%）to middle degree（59%） abnormal. Brain CT or magnetic resonance imaging（MRI）were abnormal in 53% of patients and majority of them showing that T2 or FLAIR hyperintensities within frontal lobes and temporal lobes. All patients were treated with a combination of corticosteroids and IVIg .Six patients were received immunosuppressive agent（Clclophosphamide or Mycophenolate Mofetil）. Except one was lost to follow-up, 86% experienced complete or near-complete recovery （modified rankin scale[MRS]0-2）. Conclusion The incidence of anti-NMDAR encephalitis is not uncommon in men, and this disease is less associated with tumor. Anti-NMDAR encephalitis is characterized by psychiatric-behavior abnormality, seizures and anti-NMDAR antibody positive in CSF. 79% patients received a substantial recovery by early first-line immunosuppressi

关 键 词：抗NMDA受体脑炎 临床表现 脑脊液 头颅MRI 预后 

分 类 号：R512.3[医药卫生—内科学]